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Therapeutic Effects of Dipolar Aprotic Substances on Niemann‐Pick Cells
Author(s) -
Sakuragawa Norio,
Sato Mitsuru,
Kamo Isao,
Arima Masataka
Publication year - 1987
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1987.tb00341.x
Subject(s) - acid sphingomyelinase , sphingomyelin , sphingomyelin phosphodiesterase , tetramethylurea , biochemistry , methylene , enzyme , medicine , chemistry , medicinal chemistry , membrane , solvent
Dimethylsulfoxide (Me 2 SO) increased acid sphingomyelinase activity in cultured skin fibroblasts (Sakuragawa N et al., Biochem Biophy Res Comm 1985; 126: 756–762). Various drugs were examined to find related compounds showing the same phenomenon as Me 2 SO, including other dipolar aprotic substances, various inhibitors of DNA and RNA synthesis, phorbol esters, vitamin E and its derivatives and other miscellaneous agents. Several dipolar aprotic substances, such as Me 2 SO, dimethylacetamide, tetramethylurea and hexa‐methylene‐bisacetamide, increased the acid and Mg‐independent neutral sphingomyelinase activity in human cultured skin fibroblasts. These dipolar aprotic substances also increased acid and Mg‐independent neutral sphingomyelinase activities in Niemann‐Pick cells, type C, to 300 to 700% of those of untreated controls, repairing their enzyme deficiency. Other lysosomal hydrolases increased to a slightly lesser extent than sphingomyelinase. Dipolar aprotic substances could be used as therapeutic drugs for Niemann‐Pick disease, type C.